title: Practice of noninvasive ventilation for cystic fibrosis: a nationwide survey in France.
abstract: BACKGROUND
  No guidelines are available for noninvasive ventilation (NIV) for cystic fibrosis (CF).
  
  
  OBJECTIVE
  To survey and evaluate the use of NIV for CF in France.
  
  
  METHODS
  We surveyed the coordinator physicians of every accredited CF center in France.
  
  
  RESULTS
  The respondents represented 36 centers (15 pediatric centers, 13 adult centers, and 8 centers that see both pediatric and adult patients), which had a total of 4,416 patients with CF at the time of the study, 168 (3.8%) of whom were using NIV. NIV was being used more often in the adults centers (7.6% of these patients) than in the pediatric centers (1.2% of these patients) or adult-and-pediatric centers (4.1% of these patients) (P= .01). All the respondent centers use NIV as first-line treatment for severe hypercapnic respiratory exacerbation and for stable diurnal hypercapnia, especially when associated with sleep disturbance. Bi-level pressure-targeted ventilation is the preferred ventilation mode. Settings are adjusted based on arterial blood gas values, noninvasive evaluation of patient-ventilator synchrony, patient comfort, and sometimes a sleep study. The surveyed centers reported a number of expected benefits from NIV, but few of those benefits have been proven. Problems with NIV are common and limit its use.
  
  
  CONCLUSIONS
  We found a relative homogeneity in these French centers' stated indications for and use of NIV, which highlights their numerous expectations about the benefits of NIV, which contrasts with the few validated benefits. Studies of the benefits of NIV are needed.
authors.affiliation: Pediatric Pulmonary Department, Hôpital Armand Trousseau, Centre de Ressources et de Compe'tences pour la Mucoviscidose, Université Pierre et Marie Curie-Paris 6, Paris, France. brigitte.fauroux@trs.aphp.fr
authors.forename: Brigitte
authors.initials: B
authors.lastname: Fauroux
authors.forename: Pierre-Régis
authors.initials: PR
authors.lastname: Burgel
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Boelle
authors.forename: Claire
authors.initials: C
authors.lastname: Cracowski
authors.forename: Marlène
authors.initials: M
authors.lastname: Murris-Espin
authors.forename: Raphaele
authors.initials: R
authors.lastname: Nove-Josserand
authors.forename: Nathalie
authors.initials: N
authors.lastname: Stremler
authors.forename: Laurence
authors.initials: L
authors.lastname: Derlich
authors.forename: Philippe
authors.initials: P
authors.lastname: Giovanetti
authors.forename: Annick
authors.initials: A
authors.lastname: Clément
mesh_terms: D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000628:therapy*; D005500:Follow-Up Studies; D005602:France; D019538:Health Care Surveys; D006801:Humans; D017063:Outcome Assessment, Health Care; D017410:Practice Guidelines as Topic; D012121:Respiration, Artificial / Q000592:standards / Q000706:statistics & numerical data*; D012124:Respiratory Care Units / Q000592:standards / Q000706:statistics & numerical data*; D012189:Retrospective Studies